• Logo
  • SBMUJournals

Plasma Volume to Achieve Remission in Atypical Hemolytic Uremic Syndrome

Soheila Mahdaynia, Hooman Nakysa, Hasan Otukesh, Rozita Hoseini-Shamsabadi, Azar Nickavar
21

Views

PDF

Abstract

Introduction: Atypical hemolytic uremic syndrome (HUS) is defined as a heterogeneous group of disorders. Plasma infusion or plasma exchange is the rescue therapy for this life-threatening syndrome. There is no evidence for the volume of plasma required to induce remission.
Materials and Methods: Between 2007 and 2018, Forty – two patients (M=20, F=22) with a diagnosis of recurrent or familial atypical hemolytic uremic syndrome (aHUS) who were admitted to Ali-Asgar Children’s Hospital were enrolled in this observational retrospective study. The total volume of plasma required for normalizing platelet (>150000) and LDH (<500 IU), eliminating hemolysis, and decreasing serum creatinine at first presentation of disease was calculated. Patients with TTP, vasculitis, and post infectious HUS were excluded.
Results: The mean age of the patients was 53 months (3-144 m). The majority of patients achieved remission at first presentation by plasma infusion (5 under peritoneal dialysis and 4 under hemodialysis) but ten patients required plasmapheresis. A total of 980 units of FFP perfused with a total volume of 195.975 L. The median (range) total plasma volume required for remission was 166 ml/kg (43-2850 ml/kg).

Conclusions: This study showed that the required plasma volume for the acute phase of atypical HUS for controlling the first attack of disease.

Keywords: Atypical Hemolytic Uremic Syndrome; Plasma Volume; Remission Induction; Child.


Keywords

Hemolytic uremic syndrome, Plasma infusion, children

References

Nester CM, Barbour T, de Cordoba SR, Dragon-Durey MA, Fremeaux-Bacchi V, Goodship TH, et al. Atypical aHUS: state of the art. Molecular immunology. 2015;67(1):31-42.

Noris M, Remuzzi G. Atypical hemolytic–uremic syndrome. New England Journal of Medicine. 2009;361(17):1676-87.

Loirat C, Frémeaux-Bacchi V. Atypical hemolytic uremic syndrome. Orphanet journal of rare diseases. 2011;6(1):60.

Roseff SD, Luban NL, Manno CS. Guidelines for assessing appropriateness of pediatric transfusion. Transfusion. 2002;42(11):1398-413.

Thysell H, Oxelius VA, Norlin M. Successful treatment of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura with fresh frozen plasma and plasma exchange. Acta medica Scandinavica. 1982;212(5):285-8.

Nathanson S, Frémeaux-Bacchi V, Deschênes G. Successful plasma therapy in hemolytic uremic syndrome with factor H deficiency. Pediatric Nephrology. 2001;16(7):554-6.

Licht C, Weyersberg A, Heinen S, Stapenhorst L, Devenge J, Beck B, et al. Successful plasma therapy for atypical hemolytic uremic syndrome caused by factor H deficiency owing to a novel mutation in the complement cofactor protein domain 15. American journal of kidney Diseases. 2005;45(2):415-21.

Kim JJ, Goodship TH, Tizard J, Inward C. Plasma therapy for atypical haemolytic uraemic syndrome associated with heterozygous factor H mutations. Pediatric Nephrology. 2011;26(11):2073-6.

Ariceta G, Arrizabalaga B, Aguirre M, Morteruel E, Lopez-Trascasa M. Eculizumab in the treatment of atypical hemolytic uremic syndrome in infants. American Journal of Kidney Diseases. 2012;59(5):707-10.

Köse Ö, Zimmerhackl L-B, Jungraithmayr T, Mache C, Nürnberger J, editors. New treatment options for atypical hemolytic uremic syndrome with the complement inhibitor eculizumab. Seminars in thrombosis and hemostasis; 2010: © Thieme Medical Publishers.

Schmidtko J, Peine S, El-Housseini Y, Pascual M, Meier P. Treatment of atypical hemolytic uremic syndrome and thrombotic microangiopathies: a focus on eculizumab. American Journal of Kidney Diseases. 2013;61(2):289-99.

Cho HY, Lee BS, Moon KC, Ha IS, Cheong HI, Choi Y. Complete factor H deficiency-associated atypical hemolytic uremic syndrome in a neonate. Pediatric Nephrology. 2007;22(6):874-80.

Ariceta G, Besbas N, Johnson S, Karpman D, Landau D, Licht C, et al. Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome. Pediatric nephrology. 2009;24(4):687.

Loirat C, Noris M, Fremeaux-Bacchi V. Complement and the atypical hemolytic uremic syndrome in children. Pediatric nephrology. 2008;23(11):1957-72.

Michon B, Moghrabi A, Winikoff R, Barrette S, Bernstein ML, Champagne J, et al. Complications of apheresis in children. Transfusion. 2007;47(10):1837-42.




DOI: https://doi.org/10.22037/jpn.v7i1.24213

Refbacks

  • There are currently no refbacks.